1 case of primary intracranial cartilage sarcoma

Primary intracranial cartilage sarcoma is a rare intracranial malignant tumor, which comes from abnormal residuals of cartilage cells in the embryonic period, which is difficult to diagnose the disease by imagingJilin University First Hospital received one case of right temporal logo cartilage sarcoma from December 2016 to February 20171Medical record summarymale, 57 years old, due to the medical examination of the head CT accidentally found right side of the temporal occupancy disease was admitted to hospitalCheck: No positive signsHead CT can be seen on the right side of the blade-like mixed density shadow, inside the egg shell-like calcification shadowHead MRI flat sweep visible right temporal lobe mixed long T1, mixed long T2 signal shadow, Flair mixed high signal, size 3.6 cm x 3.5 cm x 3.8 cm, lesions and right tibia part of the boundary is not clear, the surrounding brain tissue visible edema signalMRI-enhanced scanning indicating that the lesions were significantly unevenly strengthened, and the unreinforced area of no-reinforced no knots was visible insideLung CT did not see pulmonary occupancy lesions, PET-CT saw the right tibia bone damage with increased metabolism, the right temporal lobe mixed density with calcification, the rest of the body image did not see significant abnormalitiesPreoperative binding imaging data (Figure 1) consider not excluding malignant meningioma, vascular peripheral cell tumor or spinal stomaFigure 1 Open cranial surgery to remove primary intracranial cartilage sarcoma1A preoperative CT shows the right side of the cranial nest mixed density shadow; 1B, 1C preoperative enhancement MRI lesions uneven reinforcement; 1D preoperative CT reconstruction shows right tibia bone damage; 1E postoperative CT; 1F 3 months after surgery enhanced MRI No recurrence was observed; 1G postoperative pathology showed nodule growth of cartilage tissue (Sumugin-I-Red dye x 40); 1H postoperative pathology (Sumu-I-Red dyeing x 200)improved the relevant examination after the selection period of open cranial surgeryMost of the tumors seen in the surgery are located outside the epidural, gray-white, blood supply medium, slightly tough texture, tumor tissue invasion and part of the epidural bone damage, surgery to see the tumor around the brain tissue pressure and edema is obviousDuring the operation with the help of an ultrasound attractor (CUSA) to remove the tumor block all, closely repair the epidural, the end of the cranial surgery After surgery, the patient is clear, quadriplegic muscle strength V grade, pathological reflex (-) Pathological diagnosis: right temporal lomacart sarcoma I - II grade Immune grouping: Ki-6710%, CK-pan (-), EMA (-), Vimentin (-), S-100 (-), CK19, CK15, CEA, CK7, CK20, CK68 (-), GFAP (brain nerve plus), Nestin (brain nerve plus), Oligo-2 (brain nerve and dissolved enzyme Lysomezy (s) After surgery, the patient recovered well, after 3 weeks of radiation therapy, after surgery, the target absorption of the tumor bed: DT60/30f/2.0Gy Follow-up for 3 years, no recurrence 2 Discuss the origin and epidemiological characteristics of 2.1 tissue
the migration of nerve cells during the embryonic embryonic period from the back side of the neural tube to the facial protochveostion, and to form a skull in subsequent development with the middle embryo layer In the process of "cartilage bone", abnormal, immature cartilage cells remain and multiply malignantly to form cartilage sarcoma Cartilage sarcoma occurs mostly in the long bones of the limbs, the primary intracranial cartilage sarcoma is extremely rare, has an aggressive, intracranial cartilage sarcoma is good at the base of the skull age of onset, peak age 10 to 35 years old Combined with the current case reports, the incidence of the male and female ratio of about 1:3, but the reason is not known Because of its low incidence, clinical manifestations and imaging no obvious specificity, the misdiagnosis rate is high 2.2 Clinical performance
clinical manifestations of the disease are not characteristic, but can be due to its occupancy effect of limb paralysis, sensory abnormalities, vision loss, epilepsy, etc ; 2.3 diagnosis a large number of uneven calcification is a significant feature of the disease CT visible unboneized parts are equal and slightly lower density, while calcification presents a high density shadow in the clumps, and bone damage can be evaluated CTA examination can identify the disease to a certain extent with diseases such as venous malformation stoicism and less protrusion glioblastoma It is worth noting that when the tumor is located in the saddle area, it is necessary to carefully screen with craniopharyngeal tube tumors and spinal stomas containing calcified, in order to prevent misdiagnosis MrI tests can clearly identify tumor boundaries The lesions are mostly equal or long T1, mixed T2 signal, enhance duplication can be seen when the tumor peripheral strengthening, the tumor internal fibrous tissue visible strengthening, while the cystic fluid, calcified components are no obvious reinforcement, so that the internal strengthening is uneven, showing honeycomb-like reinforcement In addition, the authors found in clinical work that MRS may not be available due to the complexity of the internal composition of the tumor and the possible addition of more calcified, cystic fluids and other substances in the area of interest 2.4 pathological characteristics
cartilage sarcoma is divided into common type, interloper, de-differentiated type, transparent cell type In 2013, WHO classified common cartilage sarcoma into I, II and III according to its indicators of nuclear split image and isotype A large number of cartilage islands can be seen under the mirror as another feature of the disease EMA (-), Vimentin ( , s-100) is a feature of the original cartilage cells, while CK/EMA (-) is a typical marker of spinal stoma from the source of the outer embryo layer, which is possible to identify 2.5 treatment surgical treatment is still the preferred treatment for primary intracranial cartilage sarcoma The surgery needs to be strictly stopped by the hemorrhage, sealing the edges of the bone with bone wax and other, and firmly patching the epidural The application of an ultrasound attractor in the procedure can quickly remove most of the tumor center, then reduce the tumor volume, and then carefully peel the tumor boundary with a microoperation, and eventually completely cut the tumor, this method of excision is also applicable to other large, complex surrounding structure of intracranial tumors BLOCH and others believe that the same postoperative auxiliary radiotherapy, compared with the second total cut, the survival rate of the surgical total cut did not have a significant difference In practice, the first operation should be done to cut the tumor as completely as possible under the condition of controlling the risk of surgery and weighing the efficacy of the operation Postoperative radiotherapy can reduce the rate of local recurrence, but there is no definitive evidence of chemotherapy regimen for primary intracranial cartilage sarcoma